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Atypical primary meningioma in the nasal septum with malignant transformation and distant metastasis
Byoung Joon Baek1, Jae-Min Shin, Chi Kyou Lee
1Department of Otolaryngology-Head and Neck Surgery, Soonchunhyang University College of Medicine, Cheonan Hospital, 23-20 Bongmyung-Dong, Cheonan 330-721, Chungcheongnam-do, South Korea. bjbaek@schmc.ac.kr
Background:
Primary extracranial meningiomas (PEMs) originating from the nasal septum are extremely rare, as are extracranial metastases of meningiomas.
Case Presentation:
A 44-year-old male presented with a 2-month history of left-side nasal obstruction and frequent episodes of epistaxis. A friable mass originating from the nasal septum was resected completely via an endoscopic endonasal approach. According to WHO criteria, the tumor was diagnosed as an atypical meningioma radiologically and histopathologically. Two years later, a tumor recurred at the primary site with the same histopathological findings, and the patient was given local external radiotherapy (6840 cGy in 38 fractions). Two months after this local recurrence, a left anterior chest wall mass and a left parietal area scalp mass were observed. The subcutaneous mass was resected and showed histological evidence of malignant transformation. Several months after the last operation, the patient died.
Conclusions:
We describe the clinical, radiological, and bio-pathological features of this unique case and review the literature on atypical PEMs originating in the nasal septum. To our knowledge, this is the first reported case of an atypical PEM originating from the nasal septum that recurred with malignant transformation and extracranial metastasis.
Insights
This case report details an extremely rare atypical primary extracranial meningioma (PEM) of the nasal septum. The tumor recurred, underwent malignant transformation, and metastasized extracranially, leading to patient death.
Area of Science:
- Neuro-oncology
- Head and Neck Pathology
Background:
- Primary extracranial meningiomas (PEMs) are rare.
- Nasal septal origin for PEMs is exceptionally uncommon.
- Extracranial metastases of meningiomas are also infrequent.
Observation:
- A 44-year-old male presented with nasal obstruction and epistaxis.
- A nasal septal mass was resected and diagnosed as atypical meningioma.
- The tumor recurred locally two years later and was treated with radiotherapy.
Findings:
- Two months post-radiotherapy, the patient developed extracranial masses.
- Histological examination revealed malignant transformation of the recurrent tumor.
- This represents the first reported case of atypical PEM from the nasal septum with malignant transformation and extracranial metastasis.
Implications:
- Highlights the potential for aggressive behavior in atypical nasal septal meningiomas.
- Underscores the importance of vigilant follow-up for these rare tumors.
- Suggests a need for further research into the biological behavior and treatment of atypical PEMs.
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